Progression of disease in extra hepatic biliary atresia is associated with reduced total hepatic blood flow: descriptive results of a prospective pilot study

Hepatic fibrosis and cirrhosis develop progressively in extrahepatic biliary atresia despite timely surgical intervention. We aimed to study total hepatic blood flow [hepatic artery and portal vein flow] as a possible predictive factor of outcome of infants having biliary atresia who had underwent Kasai portoenterostomy. Twenty Infants having biliary atresia underwent colored and pulsed Doppler ultrasound studies. They were done before and 2-3 months after portoenterostomy. Hepatic artery, portal vein and single hepatic vein mean flow, mean diameter, mean velocity, hepatic arterial to portal venous flow ratio and total hepatic flow/kg were calculated and correlated to final outcome. The detected mean total hepatic flow and total hepatic flow/kg preoperatively was 685.5 +/- 296 ml/min and 147.1 +/- 51.4 ml/min/kg and post-operatively in those who became anicteric was 854.4 +/- 107 ml/min and 149.5 +/- 37.2 ml/min/kg, 539.2 +/- 337.7 ml/min and 112.1 +/- 78.6 ml/min/kg in those who developed chronic disease and in those who died was 157.6 +/- 79.6 and 30.9 +/- 16.1 ml/min/kg respectively. Unresolving cholestasis in infants having biliary atresia with poor outcome following portoenterostomy is associated with decreased post-operative total hepatic flow. Preoperative total hepatic flow did not correlate with postoperative total hepatic flow

Wilson's disease in Egyptian children: a single center 10-year experience

Wilson's disease [WD] is a multisystem disease with variable presentations. The aim of this work is to illustrate the diverse patterns of presentation of WD in Egypt. To the best of our knowledge, this is the first report regarding the clinical presentation of WD in Egypt. The study included all patients of WD presented to Pediatric Hepatology Unit Cairo University Children's Hospital within a period of ten years [1996-2005]. Analysis of date included thorough history, clinical examination, laboratory findings and treatment. Nine cases presented with hepatic manifestations [64.29%]; 2 with acute fulminant hepatitis and 7 with chronic hepatitis. Two cases presented with neurological manifestations [14.29%]. Three cases [21.4%] were presymptomatic siblings of patients with WD. The age range was 5 to 15 years. Eight patients [57.14%] had Kayser Fleischer ring [KF ring], but none had cataract. All patients had low serum ceruloplasmin level. Ten patients [71.42%] had high basal urinary copper in 24 hours and all had markedly elevated urinary copper in 24 hours after penicillamine [penicillamine challenge test]. The diagnosis of WD can be made provided that it is suspected in any patient presenting with obscure hepatic or neurological manifestations. Moreover, screening of asymptomatic relatives is a key point as 21.4% of our cases were presymptomatic relatives. Whether the predominance of hepatic presentation in our patients, was a real predominant pattern of presentation in Egypt or was due to center, awaits further studies

Safety and efficacy of rifampicin in children with cholestatic pruritus

Objective: The present study aimed at verifying the safety and efficacy of rifampicin in ameliorating pruritus in pediatric patients suffering from persistent cholestasis

Methods: Twenty-three patients attending the Pediatric Hepatology Unit at Cairo University Children's Hospital, Egypt, were included in the present study. They were suffering from intractable pruritus secondary to persistent cholestasis from various etiologies. They were 14 males [60.87%] and 9 females [39.13%]. The mean duration of itch was 19 +/- 27.5 months. Rifampicin was started at a dose of 10 mg/kg/day in two divided doses. Liver function tests were followed up weekly to detect any deterioration that may be attributed to the drug

Results: Seventeen patients [74%] showed improvement of pruritus with rifampicin. Fourteen out of the seventeen [61%] improved at a dose of 10 mg/kg/day in 2 divided doses. The remaining 3 patients [13%] needed gradual dose increase by increments of 2 mg/kg/day every 2 weeks [maximum dose 20 mg/kg/day] until clinical improvement was observed. None of the patients showed any deterioration in liver functions, even though, a significant improvement in total serum bilirubin, ALT and AST was noticed following therapy

Conclusions: Rifampicin in a dose of 10-20 mg/kg/day is safe and effective in ameliorating uncontrollable pruritus in pediatric patients suffering from persistent cholestasis. No hepatoxicity was noted on close follow up in the studied children